An 85-year-old Japanese woman had noticed erythema on her vulvar region 3 y
ears before. The erythema gradually increased in size and followed erosion
and ulceration with pigmentation on the edge of the erythema. A skin biopsy
from the pigmented area showed large round cells with ample cytoplasm, whi
ch formed nests or glandular structures. In the dermis there was mass forma
tion of basophilic cells and peripheral cells in a palisade arrangement. Th
e tumor cells in the epidermis showed positive immunoreactivity for carcino
embryonic antigen; on the other hand, the dermal tumor was negative. We dia
gnosed that the tumor in the epidermis was vulvar Paget's disease, and the
dermal tumor was a solid type of basal cell carcinoma. We speculate that th
e vulvar Paget's disease preceded and then the basal cell carcinoma develop
ed in the area of Paget's disease. This is the first report in which basal
cell carcinoma in the area of Pager's disease was documented.